22-24 Abstract Lemierre’s syndrome (postanginal sepsis/necrobacillosis) is a rare clinical entity characterised by the triad of acute oropharyngeal infection, with secondary thrombophlebitis of the internal jugular vein and metastatic septic emboli. A high degree of clinical suspicion is needed to diagnose the syndrome and the radiologist’s aware- ness of it may expedite the diagnosis and therefore the appropriate treat- ment for these potentially fatal seque- lae of a ‘sore throat’. We present a case of a 20-year-old man admitted to Helen Joseph Hospital in July 2004 with multiple lung abscesses, in whom internal jugular vein thrombophlebitis was subsequently diagnosed. Chest radi- ographic and computed tomography (CT) images accompany a brief review of this largely forgotten syn- drome . Introduction Lemierre’s syndrome is a rare form of postanginal septicaemia, usually caused by gram-negative oral anaer- obes, the most common pathogen being Fusobacterium necrophorum (necrobacillosis).1 Bacterial oropha- ryngeal infection, such as pharyngitis or tonsillitis, is followed by local inva- sion of the lateral pharyngeal space and septic thrombosis of the internal jugular vein, and finally the occur- rence of metastatic complications.2 The entity was first described in detail by Lemierre in 1936.3 Before the advent of antibiotics, Lemierre’s syn- drome was common, often following a fatal course, with a 90% mortality rate.3,4 More recently, however, the widespread use of antibiotics in the treatment of oropharyngeal infections has led to a marked decline in preva- lence and Lemierre’s has become a largely forgotten disease.3,5 The syndrome typically occurs in previously healthy adolescents and young adults, with a short interval between the oropharyngeal infection and the onset of septicaemia.6,7 The most common sites of secondary sep- tic emboli are the lungs (79.8%)2 and large joints,7 whilst less commonly documented sites include the liver, spleen and brain.1,3,8 Today the prog- nosis is relatively favourable with early, directed antimicrobial therapy.4,9 The radiologic findings in Lemierre’s syndrome are integral to its diagnosis. Case report A 20-year-old man was admitted to Helen Joseph Hospital with a 2-week history of increasing left-sided facial swelling and mild neck stiffness, followed by a productive cough of 1 week’s duration. On examination he was pyrexial, with mild neck stiffness, left-sided facial swelling and scattered crepita- tions throughout both lungs on aus- cultation. ENT and cardiac examina- tions were documented as normal. The admission chest X-ray (Fig.1) revealed multiple, bilateral, poorly defined nodules with central break- down or cavitation in the majority, a small right-sided pleural effusion and cervical soft-tissue prominence. The differential diagnosis at this stage included infectious causes, e.g. tuber- culosis, Staphylococcus, Klebsiella and septic emboli, with Wegener’s granu- lomatosis, rheumatoid nodules and metastases less likely.10 The patient had no history of rheumatic heart disease or intra- venous drug abuse. Investigations yielded the follow- ing results. Full blood count revealed a neu- trophil leucocytosis (white cell count CASE REPORT 22 SA JOURNAL OF RADIOLOGY • July 2005 Lemierre’s syn- drome – the uncommon cold R Such MB MCh, FCRad (Diag) E Joseph MB BCh, FFRad (Diag) Department of Radiology University of the Witwatersrand Helen Joseph and Chris Hani, Baragwanath Hospitals Johannesburg Fig.1 Chest X-ray demonstrates cavitating nodules, a small right-sided pleural effusion and soft-tissue fullness in the neck bilaterally. 22-24 7/30/05 6:26 AM Page 22 23.8 x 109/l) and thrombocytopaenia (platelets 39 x 109/l). The HIV ELISA was negative and the cerebrospinal fluid specimen was normal. Echocardiography and abdominal ultrasound examinations were also unremarkable. Serial sputum speci- mens were collected for MC & S and acid-fast bacillus which eventually yielded Escherichia coli, whilst blood cultures were contaminated by a coagulase-negative Staphylococcus (Staphyloccus epidermidis). Initial treatment was commenced with intravenous cefuroxime and gentamicin, a second-generation cephalosporin and an aminoglycoside respectively, for synergistic broad spectrum ß-lactam and gram-nega- tive cover.11 However, the patient remained intermittently pyrexial and described increasing neck pain and dysphagia, prompting further investigation with a pre-and post-contrast axial CT scan of the neck, utilising 100ml Jopamiron 300 at a rate of 2 ml/s (Figs 2 and 3) This revealed a small left- sided parapharyngeal abscess, ipsilat- eral internal jugular vein (IJV) throm- bosis with enhancement of the vessel wall and numerous pulmonary nod- ules in the visible lung apices, with varying degrees of cavitation. These were situated predominantly in the lung periphery and were associated with a right-sided pleural effusion. The diagnosis of oropharyngeal abscess with secondary IJV throm- bophlebitis and septic emboli was made and metronidazole was added to the existing treatment regimen in order to cover anaerobic pathogens. In addition warfarin anticoagulation was commenced. Favourable clinical response was obtained, and follow-up chest X-ray and CT scan of the neck after a 3-week interval revealed supporting radiolog- ic improvement. The abscess had resolved, the left IJV remained throm- bosed but without mural enhance- ment, and the pulmonary nodules and pleural effusion were decreasing in number and size (Figs 4 and 5). Discussion Whilst gram-negative anaerobes including Fusobacterium necropho- rum (a commensal organism) are documented as the most common pathogens associated with Lemierre’s syndrome,1-3,5-9 postanginal sepsis has been linked to other organisms including Eikenella corrodens12 and Salmonella, which may act as an oxy- gen consumer and facilitate the growth of anaerobes.13 We postulate that the Escherichia coli (like Salmonella, an aerobic, gram-nega- tive, enteric rod) cultured from the sputum of this patient, may have played a similar role in the pathogen- CASE REPORT 23 SA JOURNAL OF RADIOLOGY • July 2005 Fig.2. CT scan demonstrates a left IJV hypoattenuating filling defect and a thickened enhancing wall, as well as a small abscess lateral to the hyoid on the ipsilateral side. Fig.3. CT scan of the lung apices confirms peripheral cavitating nodules and a right pleural effusion. Fig.4. Repeat CT scan of the neck demon- strates ongoing left IJV thrombosis, without the presence of parietal contrast fixation. Fig.5. Repeat CT scan of the lung apices con- firms resolution of pulmonary nodules and pleural effusion 22-24 7/30/05 6:26 AM Page 23 esis of Lemierre’s syndrome.14 As the offending anaerobe was not definitively cultured, appropriate anaerobic antibiotic therapy was not instituted until the CT investigation documented evidence of oropharyn- geal infection and secondary internal jugular vein thrombophlebitis, thus suggesting the diagnosis of Lemierre’s. It is interesting to note that infec- tion of the parapharyngeal space may affect not only the IJV in the carotid space, but also cranial nerves IX - XII and the cervical sympathetic chain, with consequent neurologic signs and symptoms including hoarseness and dysphagia, collectively known as Villaret’s syndrome.3,15 Many patients with Lemierre’s present with nonspecific signs and symptoms ranging from acute pharyngitis to fever and malaise with the onset of systemic sepsis.3 IJV thrombosis typically causes tender- ness and swelling in the neck, along the line of the sternocleidomastoid muscle,5 however these findings may be minor and readily overlooked.3 Chest X-ray findings may be normal, or may exhibit nonspecific infiltrates early in the evolution of the syn- drome, with the classic features of sep- tic emboli developing only later.2,3 The triad of pharyngitis, a tender or swollen neck and a pulmonary infiltrate, should alert the clinician to the possibility of Lemierre’s.2 Typical associated radiographic findings may prove integral in the diagnosis. IJV thrombus may be demonstrat- ed at colour doppler ultrasound as non-compressible clot, frequently associated venous distention, and the absence of phasic flow.3 Contrast CT diagnostic signs include widening of the vein calibre, inhomogeneous or absent luminal opacification, and parietal contrast fixation.16 Chest X-ray or CT findings char- acteristic for septic emboli are multi- ple, peripheral, round and wedge- shaped opacities that progress rapidly to cavitation, often with associated pleural effusions. These features may be readily appreciated at CT, when the additional finding of a ‘feeding vessel’ sign (pulmonary artery leading to a nodule) may be seen.3,10 Radiologic investigations may therfore be instrumental in reaching a specific diagnosis of Lemierre’s. Treatment The management of Lemierre’s requires a prolonged course of high- dose, intravenously administered antibiotics, including adequate anaer- obic activity.1,3 The role of anticoagu- lation remains controversial, but may enhance resolution of the source of septic embolisation.3 If signs of sepsis persist, with prop- agation of the infected thrombus, lig- ation or excision of the IJV may be required.1,7 More recently, hyperbaric oxygen has been investigated as a potential adjunctive therapy in postanginal sepsis.17 Conclusion Today morbidity and mortality in Lemierre’s syndrome is caused mainly by a lack of knowledge of the syn- drome.18 It follows that awareness of this syndrome on the part of the radi- ologist should lead to the suggestion of Lemierre’s, whether by demonstra- tion of IJV thrombosis by ultrasound or CT, or through findings indicative of septic emboli in the correct clinical setting, as this may expedite the cor- rect treatment. 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